Repeated brain hemorrhage?

Cerebral vascular amyloidosis (CAA) is an intracranial microvascular disease caused by the deposition of amyloid in the cerebral cortex, pia mater, and the walls of small arteries.
There is no evidence of other systemic amyloidosis
.

The clinical manifestations are mainly dementia, psychiatric symptoms, and progressive and repeated multifocal bleeding
.

Cerebrovascular amyloidosis is one of the important causes of stroke in the elderly, and has now become an important cause of spontaneous non-hypertensive intracerebral hemorrhage in the elderly
.

Due to the diversity of its manifestations, it is easy to be misdiagnosed and missed in clinical practice.
Today, I will take you to learn more about this disease
.

Clinical manifestations ➤ Spontaneous intracranial hemorrhage (ICH): The most common manifestation of CAA is spontaneous intracranial hemorrhage, which mostly occurs in patients over 55 years of age
.

Bleeding characteristics: (1) Lobar hemorrhage occurs frequently, mainly affecting the superficial area of ​​the lobe
.

(2) The hematoma is large and lobulated
.

(3) It is easy to break into the subarachnoid space, and sometimes breaks into the ventricle to show intraventricular hemorrhage
.

(4) Multiple bleeding and bleeding are prone to recurrence
.

(5) Mostly fatal bleeding
.

 ➤ Dementia: About 30% of CAA patients develop symptoms of Alzheimer's, such as severe memory impairment, impaired concentration, orientation, and calculation, or mental disorders
.

Many patients have no symptoms of dementia, so the diagnosis of CAA cannot be easily ruled out in those without dementia
.

 ➤Others: ischemic infarction or hemorrhagic infarction, seizures, leukoencephalopathy, TIA,
etc.

 Pathological characteristics ➤CAA is a limited inflammatory pathological change and degeneration of brain tissue, which leads to changes in the permeability of small blood vessels in brain tissue, so that amyloid deposits in brain tissue and blood vessel walls, and finally forms cerebrovascular wall fibers Protein-like necrosis, dilation or stenosis, micro-aneurysm formation, blood vessel rupture and a series of changes
.

The changes can appear in all lobes, among which the parietal, temporal and occipital lobes are the most
.

 ➤The conventional HE staining of the vessel wall with CAA involvement showed a homogeneous unstructured, strongly eosinophilic glass-like amyloid change under light microscope, orange-red staining with Congo red, and yellow-green birefringence under polarized light microscope.
A characteristic is considered to be the most specific method for diagnosing CAA
.

 Diagnostic criteria ➤ Definite CAA-related hemorrhage: autopsy confirmed lobar, cortical, or subcortical hemorrhage; associated with CAA-related vascular disease; excluding hypertensive intracerebral hemorrhage
.

 ➤Pathologically confirmed CAA-related bleeding: Clinical and pathological examinations (hematoma evacuation or cortical biopsy) confirmed lobar, cortical, or subcortical hemorrhage; other causes of hemorrhage were excluded
.

 ➤ Probable CAA-related bleeding: age = 55 years; multifocal hemorrhage limited to lobar, cortical, or subcortical (including cerebellum) by clinical presentation and findings on MRI or CT; other causes of hemorrhage excluded
.

 ➤Possible CAA-related hemorrhage: age = 55 years; single lobe hemorrhage detected by clinical presentation and MRI or CT examination; exclusion of other causes of hemorrhage
.

 ➤ Suspected CAA-related hemorrhage: only a single lobe hemorrhage and no other cause of hemorrhage
.

 Differential diagnosis ➤ Hemorrhage caused by intracranial aneurysm, cerebral vascular malformation, and central nervous system tumors: hematoma margins caused by amyloid cerebrovascular disease are irregular; intracranial aneurysm hemorrhage is more common in single, mainly in the circle of Willis area; In the hemorrhage caused by intracranial arteriovenous malformation, malformed blood vessels can be seen in the lesion
.

 ➤Hypertensive intracerebral hemorrhage: Both amyloid cerebrovascular disease and hypertensive intracerebral hemorrhage occur more frequently in the elderly.
The former is located in the superficial area of ​​the lobe, and it is multiple; Or more common in the brain stem
.

 ➤ Occult vascular malformations (telangiectasia and cavernous hemangioma): The patient's age at onset is younger than amyloid cerebrovascular disease, and the bleeding foci of telangiectasia are hypointense shadows with a diameter of less than 2 cm, mainly in the pons and cerebellum; sponge Hemangioma can occur in any part of the brain, and a single lesion presents a "popcorn"-like change with mixed high signal in the center and low signal in the periphery
.

 Treatment and prognosis ➤Medical treatment: Currently, medical treatment of CAA focuses on preventing the symptoms caused by CAA
.

Newly diagnosed CAA should pay attention to preventing excessive anticoagulation, using antiplatelet drugs with caution, regulating blood pressure and preventing complications
.

Steroids and cyclophosphamide are advocated if vasculitis is present on both angiography and brain biopsy
.

For those with dementia, cholinesterase inhibitors, brain cell activators, antioxidants, etc.
can be applied
.

➤Surgical treatment: Surgery can improve the neurological status of CAA patients, but patients older than 75 years old, with parietal lobe hematoma or with ventricular hemorrhage, because surgery will increase the possibility of bleeding, so surgical treatment is not the first choice
.

However, if the age is less than 75 years old, the hematoma volume is ≤60ml, and the GCS score is >8 points, active surgical treatment can be considered, otherwise, conservative treatment should be considered
.

 ➤ Prognosis: CAA-related intracerebral hemorrhage has an in-hospital mortality rate of 24%, a 6-month mortality rate of 32%, an annual recurrence rate of 4.
4%, and a disease duration of 5-19 years
.

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